0.05).Two cases were lost to follow up,19 out of 21 cases were followed up.The follow-up duration was from 4 to 51 months[mean follow-up duration was(22.84?12.13) months].At last follow-up,10 cases(53%) had complete remission,4 cases(21%) had significant remission,4 cases(21%) had partial remission and 1 case(5%) had no remission.Tubulointerstitial lesions were related with prognosis(P1).Conclusions 1.The predominant clinical manifestation of primary FSGS is nephrotic syndrome in children.The not otherwise specified variant is the commonest pathological variant in primary FSGS.There is no relationship between patholo-gical types and clinical manifestations of FSGS.2.Most of the children with primary FSGS have a good short-term prognosis,but their long-term prognosis must be followed up.3.Tubulointerstitial lesions are risk factors for prognosis.

Humans ; Kidney Failure, Chronic ; therapy ; Renal Insufficiency, Chronic ; therapy

Objective To analyze the clinical features of immunoglobulin A(IgA) nephropathy(IgAN) occurring in the context of pre-vious acute glomerulonephritis.Methods The clinicopathologic features of 108 cases(77 males,31 females,aged 3.2 to 14.0 years old) of IgAN were analyzed restrospectively with the onset of acute glomerulonephritis in this department from Jan.1994 to Dec.2005.Results Of 108 cases with IgAN,10 cases were with the onset of acute glomerulonephritis. All the 10 cases presented with edema, hematuria and proteinuria.Eight cases had macroscopic hematuria, lasting for 2 days to 2 months.Four cases had recurrent macroscopic hematuria,microscopic hematuria lasting for 16 months.Urine protein qualitative +-+++ lasted for 1-8 months.Two cases had hypertension;2 cases had increasing blood uria nitrogen and serum creatinine.The increase of anti-streptolysin O(ASO) titer was detected in 5 of 9 cases.Two cases had a high titer of DNase-B antibody, and 4 cases had hypocomplementemia.Eight cases had prodromal infection with an interval time varying from 1 to 5 days.Renal biopsies revealed mesangial proliferative glomerulonephritis in 9 cases and focal segmental glomerulosclerosis in 1 case, all with mesan-gial IgA deposition.Conclusions The interval time between the prodromal infection and nephritis symptoms is mostly short in IgAN with the onset of acute glomerulonephritis, while macroscopic and microscopic hematuria remain a fairly long time or recurrently aggravate. Renal biopsy is necessary to diagnose IgAN for this kind of children.

Platelet-derived growth factor (PDGF), a potent chemotactic and mitogenic factor, is involved in the regulation of hematopoiesis and platelet production. Our studies demonstrate the presence of functional PDGF receptors (PDGFR) on human megakaryocytes/platelets and CD34(+) cells, and their ability to mediate a mitogenic response. PDGF promotes the ex vivo expansion of human hematopoietic stem (CD34(+)) and progenitor (CD41(+)) cells. More significantly, PDGF enhances the engraftment of human CD45(+) cells and their myeloid subsets (CD33(+), CD14(+) cells) in NOD/SCID mice. PDGF also stimulates in vitro megakaryocytopoiesis via PDGFR and/or the indirect effect on bone marrow microenvironment to produce TPO and other cytokines. It also shows a direct stimulatory effect of PDGF on c-Fos, GATA-1 and NF-E2 expressions in megakaryocytes. We speculate that these transcription factors may be involved in the signal transduction of PDGF on the regulation of megakaryocytopoiesis. PDGF also enhances platelet recovery in mouse model with radiation-induced thrombocytopenia. This radioprotective effect is likely to be mediated via PDGFR with subsequent activation of the PI3K/Akt pathway. It provides a possible explanation that blockage of PDGFR may reduce thrombopoiesis and play a role in imatinib mesylate-induced thrombocytopenia.
Animals ; Hematopoietic Stem Cells ; cytology ; Humans ; Megakaryocytes ; cytology ; Mice ; Platelet-Derived Growth Factor ; metabolism ; Receptors, Platelet-Derived Growth Factor ; metabolism ; Thrombopoiesis

Objective To observe the influence of Fibronectin-Thrombopoietin(FN-TPO) gene modified human bone marrow mesenchymal stem cells(MSCs) on the engraftment of cord blood hematopoietic stem cells.Methods FN-TPO gene modified human bone marrow MSCs combined with cord blood mononuclear cells(CB-MNC) were transplanted to sublethal dose treated severe combined immunodeficiency disease(SCID) mice.After transplantation,these mice were observed for 4 weeks.Peripheral blood cell counts were performed at different time point to assay the hematopoietic system status of the mice.Four weeks after the transplantation,human-sourced cell integration was assayed by flow cytometry(FCM) and polymerase chain reaction(PCR).Results One week after the cell transplantation,every main index of the peripheral blood cell counts in the gene modified group was higher than that in the control groups(P

Animals ; Drugs, Chinese Herbal ; pharmacology ; Female ; Heart Failure ; drug therapy ; physiopathology ; Hemodynamics ; Male ; Myocardial Ischemia ; drug therapy ; physiopathology ; Phytotherapy ; Rats ; Rats, Wistar ; Rhodiola

Combined Modality Therapy ; Female ; Genital Neoplasms, Female ; radiotherapy ; surgery ; Gynecology ; education ; trends ; Humans ; Medical Oncology ; education ; trends

Objective To summarize perioperative care and effect of surgical treatment of total anomalous pulmonary venous connection (TAPVC) in newborns and babies within 6 months.Methods From September 2001 to May 2013,ninetytwo consecutive patients with TAPVC received surgical correction under cardiopulmonary bypass (CPB) with moderate or deep hypothermia.The anatomic subtype included supracardiac type 48,intracardiac type 36,infracardiac type 6 and mixed type 2.There were 36 newboms.Pathological diagnosis was made by echocardiogram,computerized tomography and operative findings during operation.The option of procedure was determined by findings.Respirator assisted breathing after operation and cardiac function was maintained by a variety of positive inotropic drugs,to prevent the happening of the pulmonary hypertension crisis.Results Severely low cardiac output syndrome occurred in 8 patients and they died within 24 hours after operation.The remaining cases postoperative recover smoothly and receive postoperative follow-up about 1 month to 12 years,3 cases of postoperative pulmonary vein stenosis (PPVS) occurred.Conclusion Total anomalous pulmonary venous connection should be surgical treatment as soon as possible with satisfied effect.The key of perioperative care is maintenance of left heart function,negative balance of liquid,prevention and treatment of pulmonary hypertension.

Objective To observe the effects of soybean,selenium and spirulina on hemoglobin(Hb)of rats intoxicated with fluorine and aluminiums.Methods According to body weight,84 SD rats were randomly divided into control group,high aluminum group,high fluorine group,high fluorine-aluminum group,high fluorine-aluminium intoxicated rats strengthened with soybean group,high fluorine-aluminium intoxicated rats strengthened with selenium group and high fluorine-aluminium intoxicated rats strengthened with spirulina group,12 in each group.Rats in the control group and the high aluminum group were fed with feed containing 5.2 mg/kg of fluorine and 6.8 mg/kg of aluminum.In other groups,fluorine wag 106 mg/Kg and aluminum 19.7 mg/kg.Fluorine and aluminum concentration in the drinking water of the control group and the high fluorine group were 0.69 mg/L and 0.20 mg/L,respectively.In other groups' drinking water,these values were 0.69 mg/L and 90.2 mg/L,respectively.Ninety days later,Hb concentration of the whole blood was tested.Results Hb concentration of the control group,the high aluminum group,the high fluorine group,and the high fluorine-aluminum group were (160.8±6.3),(142.2±15.9),(156.1±4.9)and(145.2±6.2)g/L,respectively.Fluorine had an effect on the concentration of Hb(F=29.56,P<0.05).The Hb concentration of the high fluorine-aluminum group,the strengthened with soybean group,the strengthened with selenium group and the strengthened with Spirulina group were(145.2±6.2),(150.7±17.7),(156.8±14.5),(154.5±17.8)g/L,respectively.Though the concentration of Hb had increased,there was no significant difference between the four groups(χ2=3.304,P>0.05).Conclusions High-dose fluorine could cause varied decrease in the concentration of Hb.However,aluminum has neitherantagonistic effect nor synergistic effect on the Hb of fluorotic Rat.Soybean,selenium and Spirulina show a trend to increase fluorotic rat's Hb,but they has no evident antagonistic effect.

Imatinib mesylate has been commonly used in the treatment of patients with chronic myeloid leukemia (CML). However, a significant number of CML patients treated with imatinib developed thrombocytopenia, oligocythemia, granulocytopenia. It has been confirmed that imatinib not only inhibits BCR-ABL mutations, but also suppresses other tyrosine kinase receptor genes such as PDGFR, JAK2V617F and C-KIT mutations, providing an important potential of targeted therapy for myeloproliferative disease. As the PDGFR, JAK2 and C-KIT play important roles in the regulation of hematopoiesis, suggesting that imatinib may block the phosphorylation of PDGFR, JAK2V617F and C-KIT receptors, interrupt the signal transduction cascades, disrupt cell differentiation and proliferation. In this review, the application and the potential molecular mechanism of imatinib in the treatment of thrombocythemia and other myeloproliferative diseases are discussed.
Benzamides ; therapeutic use ; Humans ; Imatinib Mesylate ; Myeloproliferative Disorders ; drug therapy ; Piperazines ; therapeutic use ; Pyrimidines ; therapeutic use ; Thrombocytosis ; drug therapy